Craniovertebral junction (CVJ) tuberculosis is a rare disease, potentially causing severe instability and neurological deficits. The authors present a case of CVJ tuberculosis with atlantoaxial dislocation and retropharyngeal abscess in a 28-year-old man with neck pain and quadriparesis. Radiological evaluations showed a widespread extradural lesion around the clivus, C1, and C2. Two stage operations with transoral decompression and posterior occipitocervical fusion were performed. The pathological findings confirmed the diagnosis of tuberculosis. Treatment options in CVJ tuberculosis are controversial without well-defined guidelines. But radical operation (anterior decompression and posterior fusion and fixation) is necessary in patient with neurological deficit due to cord compression, extensive bone destruction, and instability or dislocation. The diagnosis and treatment options are discussed.